Renal amyloidosis in children

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BİLGİNER Y., Akpolat T., ÖZEN S.

PEDIATRIC NEPHROLOGY, cilt.26, sa.8, ss.1215-1227, 2011 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Derleme
  • Cilt numarası: 26 Sayı: 8
  • Basım Tarihi: 2011
  • Doi Numarası: 10.1007/s00467-011-1797-x
  • Dergi Adı: PEDIATRIC NEPHROLOGY
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Sayfa Sayıları: ss.1215-1227
  • Anahtar Kelimeler: Renal amyloidosis, Children, Autoinflammatory diseases, FAMILIAL MEDITERRANEAN FEVER, COLD AUTOINFLAMMATORY SYNDROME, JUVENILE CHRONIC ARTHRITIS, CLINICAL-FEATURES, SYSTEMIC AMYLOIDOSIS, PERIODIC SYNDROME, SECONDARY AMYLOIDOSIS, COLCHICINE TOXICITY, AA AMYLOIDOSIS, AL AMYLOIDOSIS
  • Hacettepe Üniversitesi Adresli: Evet

Özet

Renal amyloidosis is a detrimental disease caused by the deposition of amyloid fibrils. A child with renal amyloidosis may present with proteinuria or nephrotic syndrome. Chronic renal failure may follow. Amyloid fibrils may deposit in other organs as well. The diagnosis is through the typical appearance on histopathology. Although chronic infections and chronic inflammatory diseases used to be the causes of secondary amyloidosis in children, the most frequent cause is now autoinflammatory diseases. Among this group of diseases, the most frequent one throughout the world is familial Mediterranean fever (FMF). FMF is typically characterized by attacks of clinical inflammation in the form of fever and serositis and high acute-phase reactants. Persisting inflammation in inadequately treated disease is associated with the development of secondary amyloidosis. The main treatment is colchicine. A number of other monogenic autoinflammatory diseases have also been identified. Among them cryopyrin-associated periodic syndrome (CAPS) is outstanding with its clinical features and the predilection to develop secondary amyloidosis in untreated cases. The treatment of secondary amyloidosis mainly depends on the treatment of the disease. However, a number of new treatments for amyloid per se are in the pipeline.